2 resultados para ANTI-C5 COMPLEMENT ANTIBODY

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Objectives: To describe the diagnosis and treatment of a severely ill patient presenting with thrombotic microangiopathy (TMA) of unknown cause. Case presentation: An adult female presented to intensive care with abdominal pain and haemorrhagic shock, requiring reanimation. Results: Features of TMA were present, but initial plasma exchange was ineffective. Treatment with the anti-C5 antibody, eculizumab, improved laboratory parameters and organ function, albeit slowly. Eculizumab remains effective and well tolerated after 30 months of treatment. Conclusion: This case demonstrates the complexities and importance of early identification of atypical haemolytic uraemic syndrome in patients presenting with TMA.

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The authors present the case of a 65-year-old Caucasian man who had previously lived in Guinea-Bissau. The patient was diagnosed in Portugal with an anti-neutrophil cytoplasmic antibody-associated vasculitis and started on corticosteroids plus cyclophosphamide, with clinical improvement. Some months later, his general status deteriorated, iatrogenic bicytopenia developed and immunosuppressive drugs were tapered. Microbiological tests identified numerous larvae and eggs of Strongyloides stercoralis in various biological samples, and a diagnosis of hyperinfection syndrome was established. The patient was started on antihelminthic drugs but developed septic shock and died. This case highlights a rare and severe complication of immunosuppression in developed countries.